31 Days of Cystic Fibrosis 2019: Part 4 May 14th – 18th

May is Cystic Fibrosis awareness month, and once again I’ve done 31 Days of Cystic Fibrosis on Facebook and Instagram.
In 2019 I concentrated on a hidden side of CF and profiled people who are impacted by CF.

May 14th – May 18th 2019

31 Days of Cystic Fibrosis. May 14 – 16Merle Crawford: A Mother’s Story

For four and a half years, Merle Crawford took her daughter Carolyn back and forth to doctors. Despite a hospital admission for pneumonia and Gastroenteritis when Carolyn was 16 months, doctors dismissed Merle’s concerns. “One doctor thought I was fruit loop and another told me I was having babies too quickly.”
“The doctors at PMH didn’t think there was anything wrong with her because she didn’t look sick. They decided to put her on a fat-free diet, and when I went back, I told them it hadn’t made any difference.” Merle said. The next step was a sweat test, and Merle was told while they were not expecting a positive result, that someone would be in contact if there did prove to be something wrong.

That call did come, and at the resulting clinic appointment with Dr David Formby, Merle was told that Carolyn had CF. “Then David Formby turned to me and said to me ‘You realise she may only live a maximum of 16 years.’ I nearly fell off my chair.”

“They decided to take my two boys for the sweat test on them, but they were both clear. It was a relief, but the shock of Carolyn’s diagnosis remained. I’d never heard of CF.”

The next step was learning how to do postural drainage in hospital. “Carolyn got upset wanting to know why I was hitting her. She asked why I was doing this when she hadn’t been naughty. I explained to her that it was a problem with her chest. Explained that it was to help her, but in her mind, I was doing something awful to her.”

Carolyn’s diagnosis affected the whole family unit. “It was a complete change of lifestyle; you had physiotherapy in the morning and at night.
We got into a routine, the only thing I didn’t like was mixing up the powder, the stinking rotten enzyme powder. It smelt like horse pee.”

Merle asked David Formby if enzymes were available in tablet form, but he brushed her off stating that Carolyn was too young. “Let me try,” Merle said, “I gave her a demo and showed her how to take a tablet. I said hold your head back, and this big drink of water and they’ll all run down your throat. And she mastered it. I went back and said I don’t need this disgusting powder, can I have a script for the tablets?”

With the removal of the enzyme powder, the household went from one foul smell to another. Carolyn needed to inhale mucomyst by nebuliser before every airway clearance treatment. “It was pretty rank,” Merle said. “It stunk the whole house out.”

As Carolyn became a teen then an adult, the challenges became more confronting. “There were lung haemorrhages, and collapses. It was pretty frightening. I knew Carolyn was going downhill.”

When Carolyn was 26, Dr Gerard Ryan, from the CF Clinic at Sir Charles Gairdner Hospital, suggested she should meet with the transplant teams from either Sydney or Melbourne. As the team from St Vincent’s in Sydney, led by Dr Allan Granville was in Perth, it was an ideal starting point.

Allan Granville told Carolyn “I estimate you have about two years left to live.” “It was like a bowling ball coming across the desk whacking you fair in the chest,” Merel said. “And as it turned out, he was spot on.”

Instead of meeting with the Melbourne team for a second opinion, Carolyn put her faith in the team from Sydney. Both Merle and Carolyn moved to Sydney to start the long wait.

Merle couldn’t speak highly enough of the team from Sydney, the only hiccup being the need to navigate between two hospitals. The CF Clinic was held at RPA while St Vincent’s was responsible for both pre and post lung transplant.

After Carolyn had been on the waiting list for two years, Merle was given information that no parent wants to hear. Unless a transplant came through that weekend, the doctors would need to move Carolyn to ICU and intubate her. That would be the point of no return, for the team at St Vincent’s would no longer consider Carolyn a viable candidate for transplantation.
“I was freaking, but I didn’t let her know.”

On Sunday Carolyn and Merle received the news they had been waiting for. The next period was frantic as they prepped Carolyn for surgery. Merle said, “Carolyn asked for the room, and she said, ‘No matter what happens Mum you’ve been my rock. If I don’t come back from this, don’t be sad, because it will be a relief. But if I do – watch out!’”

“Saying goodbye to let her go to the theatre was hard. Waiting to see her again was hard. But when I did, Carolyn held up her pink fingernails and pointed to her pink lips. She was as happy as Larry.”

Allan Granville warned Merle that as Carolyn’s heart and other organs had worked so hard to keep her alive, one of the consequences would be a longer than normal recovery. “Yet sixteen days later, we were out. And I was in big trouble – Carolyn told me off for walking too slow. It was the first time she’d ever told me to hurry up.”

In November this year, Carolyn will celebrate her silver anniversary – 25 years with her new lungs.

31 Days of Cystic Fibrosis. May 17The Financial Burden of Cystic Fibrosis

Sales usually invoke images of shopping bargains. The sale I went to this week wasn’t nearly as exciting – it was at the Chemist. Instead of a bag full of fancy clothing, I spent $362 in exchange for a bag full of vitamins; usual RRP is $624, that will only cover my needs for the next three months.
I reached the threshold for the Safety Net last week. The Safety Net protects families from the high cost of medications, and for me, it means a reduced price for medications covered by the PBS until the end of the year.
Vitamins, and other over the counter medications, while not covered by the PBS are not only prescribed by my medical team, but essential given my issues with malabsorption due to CF.

For me, the alternative would be regular admissions as a day patient for infusions of both iron and magnesium. Taking calcium every day has helped my bones stabilise, putting a hip replacement on the back burner – for now.
My twice daily dose of zinc and Insolar have reduced the number of skin cancers I’ve needed removed.

These costs make having a Health Care Card essential – but many people with CF don’t have one. Currently, parents of children with CF can access the Carers pension, which provides a Health Care Card, until their child is 16. IIf studying, a child with CF may have access up until the day they turn 25.

Adults with CF can access a Health Care Card via Centrelink only as a low-income earner, or via the Mobility Allowance.

We’re lucky to live in Australia with the subsidies provided by the PBS. However, the cost of medications, both on script and over the counter, can be a crippling burden for some families living with CF.

I’m lucky to be in a position where I don’t have to make choices about paying the rent or paying for my medications – for others; tough choices are made daily.

Maggie has to hit the ground running at the beginning of the year, as her time is consumed with presenting education sessions on CF to school staff. This year, Maggie presented her sessions to over 400 teaching staff – discussing CF, how it impacts the child, their family and what the subsequent impact may be on the classroom, including how to adapt the classroom environment for a child with CF.

Additionally, Maggie presents education sessions to students. After an overview of CF, Maggie presents a lesson – either on genetics or how to stop the spread of germs via hand-washing and proper hygiene.

Recently CFWA purchased a product that is visible under UV light, and in her introduction, Maggie asks all the students to introduce themselves while holding a pre-prepared tennis ball. “They’re always excited to say their name and favourite colour. Once we’ve got around the room, I tell them I’ve tricked them, that the ball has fake germs, so we turn off the lights, and use a UV flashlight. They hold out their hands so they can see all the ‘germs’ on their hands.”

This visual demonstration is a very effective tool to show children how germs spread rather telling them. The next step is for the children to wash and dry their hands using a variety of techniques. Then the UV light is again used to demonstrate how effective, or not, different hand washing techniques can be.

Another aspect of the education component of Maggie’s job is continuing to develop and promote CF Smart. CF Smart aims to improve the understanding of CF in a school-based environment and has sections for Teachers (including eLearning), Parents, Students and Health Professionals. The website is packed full of fact sheets, tips and checklists. The eLearning components of CF Smart, which allows teachers to access a Professional Development certificate, are unique.

With CFWA’s vision, “Lives Unaffected by CF” written on her whiteboard, Maggie has a constant reminder of how her work to help teachers understand the impact of CF on the family, can subsequently make school less of a struggle for students with CF.